involuntary movements

INVOLUNTARY MOVEMENTS

Medical Unit-1 GMC,Surat

But the expression of a well-made man appears not only in his face, It is in his limbs and joints also, it is curiously in the joints of his hips and wrists. It is in his walk, the carriage of his neck, the flex of his waist, and knees... To see him pass conveys as much as the best poem,perhaps more. —Walt Whitman (1819–1892)

The basal ganglia are group of nuclei located subcortically which is part of a neuronal network organized in parallel circuits. The “motor circuit” is most relevant to the pathophysiology of movement.

Abnormal increment or reduction in the inhibitory output activity of basal ganglia give rise, respectively, to poverty and slowness of movement - Hypokinesias (i.e., Parkinson's disease) or dyskinesias.

It includes the corpus striatum (caudate nucleus and the lenticular nucleus, which includes the putamen and the globus pallidus) and other subcortical allied nuclei such as the subthalamic nucleus (STN), substantia nigra (SN) [consisting of the pars compacta (SNc) and pars reticulata (SNr)], and, more recently, the pedunculopontine tegmental nucleus (PPTg).

Currently, the putamen and caudate are together referred to as the striatum.

Basics

2 www.medicalgeek.com - vitrag24

Coronal view of the brain, showing the main basal ganglia nuclei.

Obeso J A et al. Physiology 2002;17:51-55


Summary of the main connections of the “motor circuit” of the basal ganglia.

Obeso J A et al. Physiology 2002;17:51-55


Outline

1. Overview of approach to the clinical

problem

2. Definition of movement types

3. Elements of history

4. Physical examination

5. Detailed discussion about each movement type


Broadly . . .

Hyperkinetic Movements Hypokinetic Movements


Or More Specifically . . .

Hyperkinetic Movements • Tremor (ET-Most common) • Chorea/Athetosis • Dystonia • Ballism • Myoclonus • Tics • Ataxia • Myokymia • Myorrhythmia • Restless Legs • Hyperkplexia (startle response) • Akathesia

Hypokinetic Movements

• Parkinsonism (2nd most

common)

• Apraxia

• Hesitant gaits

• Hypothyroid slowness

• Rigidity


Physiological synkinesia

e.g. Bell’s phenomenon, arm swing etc.

Myoclonic jerk

Hypnogogic myoclonus

Benign fasciculation without other signs of LMN leison

Physiological tremor low amplitude, high frequency

Physiological Involuntary movements


Bradykinesia/Akinesia

• Decreased movement • Common to parkinsonism

• 6 Cardinal Features of Parkinsonism:

– Tremor at rest – Bradykniesia/Hypokinesia/akinesia – Rigidity – Flexed posture of the neck, trunk and limbs – Loss of postural reflexes – Freezing


Types of Parkinsonism

• Parkinson’s Disease (most common

encountered by neurologists)

• Drug-induced Parkinsonism (probably the most common overall)


Features Suggestive of Atypical PD


Asynergia/Ataxia

• Asynergia refers to decomposition of movement due to breakdown of normal coordinated execution of a voluntary movement

• One of the cardinal features of cerebellar disease

• Frequently accompanied by: – Dysmetria (misjudging of distance) – Hypometria – Hypermetria

• The ataxic gait is typified by unsteadiness with a wide base, body sway and inability to tandem walk


Tremor

• Tremor consists of alternating contractions of agonist and antagonist muscles in an oscillating, rhythmic manner.

• Distinction between rest-pill rolling, postural, action or with intention or task specific

• Froment maneuver : Voluntary head movement enhances the parkinsonian rest tremer.

• Holmes’ or Rubral tremor : due to leison of red nucleus, rest + postural + kinetic tremer


•Probably the most common movement disorder. •Hallmark: postural and action tremor affecting the hands, head and/or voice. Presents as a rhythmic tremor (4–12 Hz). Typically bilateral, accentuated with goal-directed movement (Action tremor) as opposed to rest tremor (PD) & responds to alcohol. •Often, it is inherited as an autosomal dominant trait with the tremor becoming apparent by middle age and sometimes as early as childhood. •Diagnosis-Clinical grounds; No gene or identifiable pathology has been identified for essential tremor. Abnormalities of the basal ganglia, the cerebellum, the thalamus, the connections between these structures, or a combination of factors may be causative.

Essential Tremor

Katherine Hepburn


Treatment •First line- beta-blockers (propranolol 30-240 mg/d), or anticonvulsants primadone (start with 12.5mg mg/d,increase upto 125-250mg tid). Second line-benzodiazepines and methazolamide (Carbonic Anhydrase Inhibitor). Finally if all else fails- Botox or Surgical therapies such as electronic stimulation of the VIM nucleus of the thalamus or ablation of this structure (thalamotomy), can be effective treatments for those refractory to drug therapy. Can be confused with: •Physiologic tremor -physical exertion, hyperthyroidism, acute hypoglycemia, and other physical and metabolic stressors. •Induced tremor -Stimulant drugs (including caffeine and amphetamines), antidepressants, and agonist drugs (used to treat asthma).


•Chorea, athetosis, ballism & dystonia : Non-rhythmic involuntary movements may be combinations of fragments of purposeful movements & abnormal postures.

•All due to imbalance of activity in the complex basal ganglia circuits.

• Sometimes also known as

“ extrapyramidal disorders”

•Primarily conditions related to excessive dopaminergic activity in the basal ganglia.


Ballismus, Chorea, Athotosis and Dystonia

These should NOT be thought of as separate entities amenable to specific definition but rather as a SPECTRUM of movements that blend into one-another

WHY?


Because……..

They often co-exist

Even neurologists may often not be able to agree as to how a particular movement should be classified!


The spectrum

Ballismus Dystonia Tic Chorea Athetosis

Movements become - Less violent / explosive / jerky

- Smoother and more flowing

- More sustained

They differ from tics in that they cannot be suppressed by voluntary control

Myoclonus


Ballism More dramatic ballistic movements of of the arms & legs on one side

of the body (unilateral) and therefore use term “ HEMIBALLISMUS”.

Large amplitude choreic movements of the proximal parts of the limbs, Usually Unilateral,Violant & flinging movements

Treatment with antipsychotics is often effective.

Most common cause of hemi-ballism:

CVA in contralateral subthalamic nucleus

Second most common cause of ballism: Overdose of levodopa


Stereotypy

• Purposeless voluntary movements carried out in a uniform fashion at the expense of other activity

• Refers to coordinated movements that repeat continually and identically

• Resemble motor tics but there is no driving urge

• Often repeat themselves in a uniform, repetitive fashion for long periods of time


Tics

• An abrupt, brief recurrent jerky non-rhythmic stereotyped seemingly purposeless movement (motor tic) or sound (vocal tic) that is temporarily suppressible by will power

• Vary in severity over time • Usually preceded by uncomfortable feeling or

sensory urge that is relieved by carrying out the movement

• “unvoluntary” • May be simple or complex


•Neuropsychiatric condition characterized by the childhood onset of multiple motor and vocal tics-Repetitive semi-purposeful movements as blinking, winking, grinning or screwing up of the eyes. Coprolalia (the inadvertent utterance of obscenities), echolalia (involuntary repetition of other's phrases), palilalia (involuntary repetition of one's own utterances) and echopraxia (involuntary mimicking of the action of others). Worsen under stress! •Distinguished from other involuntary movements by the ability of the patient to suppress their occurrence, at least for a short time. •Often-times, affected individuals have co-existing obsessive-compulsive disorder, learning disabilities, hyperactivity/attention deficit disorder and behavioral problems. •Although the pathogenic basis is not understood, Tics are believed to result from dysfunction in the thalamus, basal ganglia, and frontal cortex of the brain, involving abnormal activity of the brain chemical, or neurotransmitter, dopamine therefore Tics can be treated with dopamine receptor blocking or dopamine depleting drugs such as neuroleptics. •Treatment of tics is symptomatic with haloperidol and other neuroleptics. •Some patients benefit from Clonidine, or Guanfancine an -2 agonist, or with benzodiazepines. •May be transmitted as an autosomal dominant trait

Tourette’s Syndrome (tics)


Tourette’s Syndrome (tics)


Tourette’s Syndrome Criteria

• Both multiple motor tics and one or more phonic tics must be present at some time during the illness, although not necessarily concurrently

• Tics must occur many times a day, nearly every day, or intermittently throughout a period of more than one year

• Anatomical location, number, frequency, type, complexity, or severity of tics must change over time

• Onset of tics before the age of 21 years (the DSM-IV criteria require onset of tics before age 18)

• Involuntary movements and noises must not be explained by another medical condition

• Motor tics, phonic tics, or both must be witnessed by a reliable examiner at some point during the illness or be recorded by videotape or cinematography


Causes of Tics


Chorea(latin choreus, dance) • Describes a class of involuntary, irregular,

purposeless, nonrhymic, abrupt, rapid, unsustained movements that seem to flow from one body part to another

• Characteristically unpredictable in timing, direction and distribution

• Can be partially suppressed into semi-purposeful voluntary movements

• e.g. an involuntary movement of arm towards face may be adapted to look-like an attempt to look at watch

• Prototypical example is Huntington’s disease • Treated with dopamine blocking agents,

carbamazepine, valproic acid. Usually self limiting. • Sex hormons for chorea gravidarum


Athetosis is a slow continuous stream of slow, sinuous, writhing movements, typically of the hands and feet. Most commonly seen together with chorea in dyskinetic motor fluctuations in PD. Also in athetoid cerebral palsy where damage occurs in the basal ganglia. Related to excessive dopaminergic activity. In PD reducing dopaminergic drugs alleviates. If Athetosis becomes faster, it sometimes blends with chorea, ie choreoathetosis/ 'choreo-athetoid' movements. Can be thought of as an athetoid movement that “gets stuck” for a period of time; thus, a patient with choreoathetosis may perform an involuntary movement in which his hand and fingers are twisted behind his head. He may hold this position for a few moments before his hand moves back in front of his body. The part of the movement when the limb was held, unmoving, in an abnormal position would be considered a dystonia (may occur alone).

Athetosis

• Pseudoathetosis = distal athetoid

movements of the fingers and toes due to loss of proprioception


Dystonia

• An abnormal movement characterized by

sustained muscle contraction, frequently

causing twisting, and repetitive movements or abnormal postures

• Tend to be patterned (in the same muscles)


Classification of Dystonia

• Primary Vs Secondary • Focal – Most common

– When a single body part is affected

• Segmental – Involvement of 2 or more contiguous regions of the

body are affected

• Multifocal – Involves 2 or more regions, not conforming to

segmental or generalized dystonia

• Generalized – Movements of one or both of the legs, trunk and

some other part of the body


Common types of Dystonia

Benign paroxysmal torticollis in infancy

Dopa responsive dystonia

Idiopathic torsion dystonia (Oppenheim’s dystonia)

Transient idiopathic dystonia of Infancy

Secondary dystonia:

1.Structural brain damage :Hemidystonia 2. Metabolic dystonia: Glutaric aciduria Wilson’s disease, Lesch Nyan syndrome Homocystinuria.


Examples of focal dystonias Blepharospasm means the involuntary contraction of the eyelids,

leading to uncontrollable blinking and closure of the eyelids. Affects women> men

Torticollis, commonly called wry neck, is the condition of spasm affecting the muscles of the neck, causing the head to assume unnatural postures or turn uncontrollably. Spasmodic torticollis, also known as cervical dystonia, is the most common of the focal dystonias.

Writer’s cramp:

- Dystonic posturing of arm when hand used to perform

specific tasks e.g. writing, playing piano

Blepherospams+ Oromandibular dystonia = Meige syndrome(Orofacial Dystonia)


Anticholinergics (benzatropine), antihistamines (diphenhydramine), anti-Parkinsons agents (trihexyphenidyl), and muscle relaxers (diazepam) & GABAB receptor agonist (baclofen).

The drug resistant dystonias can be treated by botulinum

toxin injection to the responsible muscles, to overcome the abnormal distribution of muscle activity for a period of time.

Any patient with childhood onset dystonia should receive levodopa trial to rule out dopa responsive dystonia.

Dystonia Treatment:


Dystonia-drug induced

Acute dystonic reaction- occulogyric crisis, sustained upward deviation of the eyes +/- toricollis, jaw opening, tongue protrusion and anxiety.

Usually a result of administering dopamine receptor blocking drugs, generally metaclopramide (antiemetic and gastroprokinetic agent used to treat nausea and vomiting, and to facilitate gastric emptying) or prochlorperazine (neuroleptic; antiemetic) in young. 50% in 48hrs, 90% in 5 days.

Treatment anticholinergic drugs, usually benztropine 1-2mg IV


Akathitic Movements

• “Unable to sit still”

• Feeling of inner, general restlessness that

is reduced or relieved by moving about

• Complex and usually stereotyped

movements

• Can be both generalized and focal

• Can usually be briefly suppressed

• Most common cause is iatrogenic


Restless Leg syndrome – Ekbom’s syndrome

The four core symptoms required for diagnosis are as follows: an urge to move the legs, usually caused or accompanied by an unpleasant sensation in the legs; symptoms begin or worsen with rest; partial or complete relief by movement; worsening during the evening or night.

Primary RLS is usually genetic.

Secondary RLS may be associated with pregnancy or a range of underlying disorders, including anemia, ferritin deficiency, renal failure, and peripheral neuropathy.

Most RLS sufferers have mild symptoms that do not require specific treatment. If symptoms remain intrusive, low doses of dopamine agonists, e.g., pramipexole (0.25–0.5 mg) and ropinirole (1–2 mg), are given 1–2 hours before bedtime 45 www.medicalgeek.com - vitrag24

Hemifacial Spasm

• Unilateral facial muscle contractions

• Continual, rapid, brief, repetitive spasms

• Can evolve into sustained tonic spasms

• Can often be brought out when patient

voluntarily and forcefully contracts the facial muscles


Myoclonus

• Sudden, shock-like involuntary movements caused by muscular contractions or inhibitions

• Can occur at rest or during activity (action myoclonus)

• Can be both arrhythmic or rhythmic

• Numerous classification schemes

• Differs from tics by interfering with normal movement & not suppressible.


May be caused by active muscle contraction

- positive myoclonus

May be caused by inhibition of ongoing muscle activity

- negative myoclonus ( eg. Asterixis )

Generalised - widespread throughout body

Focal / segmental – restricted to particular part of body

Hypnogogic jerk – Normal during falling sleep or waking up

Treatment:Valproic acid is drug of choice May respond to benzodiazepines-clonazepam , piracetam, primidone.

Myoclonus


Myoclonus Classification Schemes

• Clinical – Spontaneous – Action – Reflex

– Focal – Axial – Multifocal – Generalized

– Irregular – Repetitive – Rhythmic

• Pathophysiology Cortical

Focal Multifocal Generalized

Thalamic Brainstem

Reticular Startle Palatal

Spinal Segmental Propriospinal


Myokymia

• Fine quivering or rippling of muscles

• Most commonly occurs in facial muscles

• Distinguished from benign fasciculations on

EMG which shows rhythmic discharges in

doublets and triplets

• Causes: Pontine lesions (MS), Pontine

gliomas


Myorhythmia

• Term used to describe a slow frequency

(<3Hz), prolonged, rhythmic or repetitive

movement which does not have the sharp

movement of a myoclonic jerk

• Typically described in context of Whipple’s disease


Paroxysmal Dyskinesias

• Refer to dyskinetic movements that appear suddenly and then disappear after a variable period of time

1. Paroxysmal kinesigenic dyskinesia - Triggered by sudden movement and lasts seconds to

minutes - Can be hereditary or symptomatic - Can be dystonic, ballistic or choreic

2. Paroxysmal nonkinesigenic dyskinesia - Often familial, triggered by stress, fatigue, caffeine or

alcohol - Lasts minutes to hours

3. Episodic ataxias


Involuntary movements as facial grimacing , chewing movements, tongue movements (oro-bucco-mandibular dyskinesia).

Appear after weeks, generally years of exposure to dopamine receptor blocking drugs. Older or classical ‘typical’ antipsychotics e.g. chlorpromazine, haloperidol

With typical antipsychotics prevalence around 20%

Thought to be due to receptors super sensitivity to these drugs . Changes in synapse number and dendrite arborisation also occur.

Tardive dyskinesia


DEEP BRAIN STIMULATION


Let’s Talk About History


Elements of the history

• Time course/functional disability/effect upon quality of life

• Past medical history, including infections and toxin exposures

• Drug history (current, previous, recreational) • Alcohol responsiveness • Family history • Neuropsychiatric features • Autonomic symptoms • Sleep problems


Other elements of history

• Do specific actions provoke the movement?

• Do the movements occur in relation to other

actions?

• Do the movements occur during sleep?

• Are there any associated sensory symptoms?

• Can the movements be suppressed?

• Are there aggravating or alleviating factors?


Points to remember on exam

• Observe casually during history:

– Any involuntary movements and their distribution

– Utterances and vocalizations

– Blink frequency

– Excessive sighing

• Cognitive assessment

• Cardiovascular – orthostatics, limb colour

• Gait, axial tone


Points to remember on exam..con’d

• Eye movements (range, speed)

• Limb examination (writing, hand posture)

• Tremors/postures

• Tone

• Power and co-ordination

• Fine finger and rapid alternating

movements

• Reflexes/plantars


Observation

• Rhythmic vs. arrhythmic • Sustained vs. nonsustained • Paroxysmal vs. Nonparoxysmal • Slow vs. fast • Amplitude

• At rest vs. action • Patterned vs. non-patterned • Combination of varieties of movements

• Supressibility


Rhythmic vs Arrhythmic

Rhythmic

• Tremor

• Dystonic tremor

• Dystonic myorhythmia

• Myoclonus (segmental)

• Myoclonus (oscillatory)

• Moving toes/fingers

• Periodic movements of sleep

• Tardive dyskinesia (stereotypy)

Arrhythmic

• Akathitic movements

• Athetosis

• Ballism

• Chorea

• Dystonia

• Hemifacial spasm

• Hyperekplexia

• Arrhythmic myoclonus

• Tics


Paroxysmal vs. Nonparoxysmal

Paroxysmal

• Tics

• PKD

• PNKD

• Sterotypies

• Akathic

movements

• Moving toes

• Myorhythmia

Continous

• Abdominal dyskinesias

• Athetosis

• Tremors

• Dystonic postures

• Myoclonus, rhythmic

• Tardive sterotypy

• Myokymia

• Tic status 63 www.medicalgeek.com - vitrag24

Hyperkinesias that persist during sleep

• Secondary palatal myoclonus • Nocturnal Myoclonus (Periodic Limb

Movement Disorder) • Ocular myoclonus • Oculofaciomasticatory myorhythmia • Fasciculations • Moving toes • Myokymia • Neuromyotonia • Ressless leg syndrome


Refereces:

Dejong’s Neurological Examination – 7th Ed

DeMeyer’s Neurological Examination – 6th Ed

Localization in cinical neurology : Paul Brazis – 6th Ed

Harrison’s PRINCIPLES OF INTERNAL MEDICINE : Eighteenth Edition

UpToDate (http://www.uptodate.com)

eMedicine (http://www.emedicine.com)

The Basal Ganglia and Disorders of Movement: Pathophysiological Mechanisms : American Physiology Society

Bates’ guide to physical examination & history taking – 11th Ed


http://www.emedicine.com/

That’s all folks! Thank You for listening!


involuntary movements

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